Acute lymphoblastic leukaemoa - see paeds? Flashcards
What is acute lymphoblastic leukaemia
a malignancy of precursor cells from the lymphocyte lineage, the most common of which is B-cell
Most common childhood malignancy
Red flags for ALL
Persistent fever
Pallor
unusual bleeding
Lymphadenopathy
Organomegaly
Cause of ALL
Genetic - indrease risk among monozygotic twins, trisomy 21, kleinfelter syndrome and fanconi anemai
Viral exposure
Smoking or radiation exposure
Pathophysiology ALL
Precursor cells chromosomal mutations arrest development -> continue clonalm expansion and more mutations -> uncontrolled proliferation of lymphoblasts -> bone marrow and organ infiltration, restrict haematopoiesis and supress bone marrow
ALL classifications
B cell lineage
T cell lineage
Rare cases NK cell lineage
Why use cytogenetic and molecular testing in B-ALL when symtpoms the same
Different subtypes have different risk - stratify risk and determine agression of treatment use
Prognostic
Examples of ALL classification
Philadelphia chromosome
Hyperdiploid - B-ALL/LBL
Hypodiploid B-ALL/LBL
T(v11q23.3) KMT2A rearranged
Clinical features ALL
Hepatomegay or spenomegaly ->anorexia, weight loss, abdo pain, distension
Fever - persistent, recurrent or refractort, night sweats, weight loss
Lymphadenopathy
Haematological = unusual bleed or petechial rahs, anaemia
MSK pain - bone pain - limp, refusal to weight bear
All present around 50% cases
Lymphadenopathy in ALL
Persistent or progressive painless firm rubbery ,ymph node
Less common clinical features ALL
Headache - ICP, N+V, irritability, meningism or focal neuro deficits
Testicular enlargement - more likely in relapse
Mediastinal mass - T cell lineage -> SVCc compression
What can present with SVC compression and what does that looj like
T cell lineage ALL due to mediastinal mass
Dysphagia, SOB, oain, swelling of face and upper limbs
FBC in ALL
Thrombocytpoenia
Anaemia
WCC - low (50%), high (20%), normal if bone marrow not supressed yet
Depends if arrested post or pre maturation
Neutropenia most likely
What investifations are required in ALL to screen for complications
Infection screen
Coag profile
U+Es, LDH, uric acid
ALL further investigations
Peripheral blood smear
Bone marrow aspiration
Can do - lymph node biopsy, CSF w LP
What investigations need to diagnose ALL
Immunophenotyping and cell morphology
When do lymph node biopsy in ALL
lymphadenopathy presenting feature - easier to see abnormal cells
What do all children with ALL have due to poor CNS involvement prognosis
CSF to evaluate for involvement
Prophylactic intrathecal chemo
What are the potential problems with a peripheral smear
May be normal if malignancy confined to bone marrow on presnetation
What use cytogenetics for in ALL
Classification of ALL to subtype WHP
Differentials for ALL
Burkitts lymphoma
AML
Aplastic anaemia
ITP
Reactive lymphocytosis
ALL vs AML
Clinically and morphologically indistinguishabel
EXCEPT ALL -> testicular and CNS involvement
AML -> skin and gum or mucous membranes
Use cytogenetics and immunophenotyping of bone marrow to confim
AML - meyloid features and antigens and absence of lymphoid antiens
Aplastic anaemia differentiate ALL
Absence of blast cells
Waht is reactive lymphoytosis
Non specific features similar to ALL
seroloy and iral tests, cultures and normal haematopoiesis
eg infectious monoculeosis
Pertussis
HIV
osteomyelitis
TB
Inital management ALL prior to starting therapy
Treat any infection, metabolic complication and transfusion if indicated
5-7 dyas of corticosteroids
hYDRATION
aLLOPUIRONAL
CNS prophylacis intrathecal
Baseline U+Es, LFTs, ECHO function
Which chemo is cardiotoxic in heam
Anthracycline
Complications of ALL
Pancytypenia (TP, anaemia, neutropenia (sepsis))
Leukostasis >100 lymphoblasts
What need to regularaly monitor in ALL
FBC - identify low levels and need for correction or prophylaxic antibiotics
Treatment leukostasis
Leukopheresis
ALL complications from therapy
Tumour lysis syndrome - induction therapy
GI toxicity - nause, mucositis, diarrhoea, const, abdo pain
Coticosteroid -related avascular necrosis
L-asparaginase-ralted coagulopathy
Anthracycline related cardiotoxicity
What chemo drug can cause coagulopathy
L-asparaginase
pFibrinogen and anti-thrombin 3 depletion
Factors -> improved prognosis in ALL
Younger patients >adults
Low WCC > high at diagnosis
T-cell worse than B-cell
Chromosomal abnormalities negatively affect - Ph+, IKFZ1
